The Many Faces of Cardiac Sarcoidosis

Virian D. Serei, MD, PhD; Billie Fyfe, MD

Disclosures

Am J Clin Pathol. 2020;153(3):294-302. 

In This Article

Prognosis

In patients with sarcoidosis, cardiac involvement confers a worse prognosis than in those without cardiac involvement.[4,5] However, prognosis is also dependent on treatment and the extent of cardiac involvement.[4] A study by Yazaki et al[31] showed that patients treated with corticosteroids from the onset of diagnosis had a 5-year survival rate of at least 75%, compared to a 5-year survival rate of 10% in untreated patients. They also showed that survival was related to heart function. Treated patients with left ventricular ejection fraction (LVEF) of 50% or higher had a significantly better 10-year survival rate of 89% as compared to treated patients with LVEF less than 50%, who had a10-year survival rate of 27%.[31]

Results are mixed when comparing the survival of cardiac sarcoid patients who receive cardiac transplants vs those who have received cardiac transplants for other conditions.[18] Akashi et al[29] showed a trend towards lower survival rates after cardiac transplantation in patients with cardiac sarcoidosis vs those without cardiac sarcoidosis. Zaidi et al[32] showed better 1-year posttransplant survival for cardiac sarcoidosis patients compared to noncardiac sarcoidosis patients. Some groups show no differences in postcardiac transplantation between cardiac sarcoidosis patients and noncardiac sarcoidosis patients.[30,33] Furthermore, the use of mechanical circulatory support as bridge to transplantation does not appear to affect survival of cardiac sarcoid patients vs noncardiac sarcoid patients receiving cardiac transplants.[30] Common causes of mortality in cardiac sarcoid patients receiving transplantation include infection, hemorrhagic complications, and acute rejection.[33] Recurrence of cardiac sarcoidosis is uncommon with rates ranging from 0% to 14%.[18]

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